Földeák Dóra, Kormányos Árpád, Domsik Péter, Kalapos Anita, Piros Györgyike Á, Ambrus Nóra, Ajtay Zénó, Sepp Róbert, Borbényi Zita, Forster Tamás, Nemes Attila
Division of Haematology, 2nd Department of Medicine and Cardiology Center, Medical Faculty, Albert Szent-Györgyi Clinical Center, University of Szeged, Szeged, Hungary.
2nd Department of Medicine and Cardiology Center, Medical Faculty, Albert Szent-Györgyi Clinical Center, University of Szeged, Szeged, Hungary.
Rev Port Cardiol. 2017 Dec;36(12):905-913. doi: 10.1016/j.repc.2017.06.014. Epub 2017 Dec 9.
While cardiac amyloidosis (CA) is a rare systemic disease characterized by extracellular deposition of protein-derived fibrils, hypertrophic cardiomyopathy (HCM) is histopathologically characterized by myocyte hypertrophy and disarray, interstitial fibrosis, and small intramural coronary arteriole dysplasia. The aim of the present study was to compare left atrial (LA) volumetric and functional characteristics between light-chain (AL) CA and HCM by three-dimensional (3D) speckle-tracking echocardiography (STE).
The AL-CA group initially consisted of 17 patients with AL-CA, but one patient was excluded due to inadequate image quality, and so the study population consisted of 16 patients (mean age: 64.0±9.6 years, five men). Their results were compared with data on 20 age-matched HCM patients (mean age: 59.8±5.2 years, 10 men) and on 16 age-matched healthy controls (mean age: 58.2±7.2 years, six men). Complete two-dimensional Doppler echocardiography and 3D-STE were performed in all cases.
Significantly increased LA volumes were observed in both AL-CA and HCM compared with the control group. Only active atrial emptying fraction was found to be significantly reduced in AL-CA patients compared to controls. Peak global and mean segmental circumferential, longitudinal and area strains showed significant reductions in AL-CA patients compared with controls, but only peak mean segmental longitudinal strain differed significantly between HCM patients and controls. While no differences were demonstrated in global and mean segmental strain at atrial contraction between HCM patients and controls, AL-CA patients showed reductions in certain strain parameters compared to controls and HCM patients.
Different patterns of LA functional characteristics were demonstrated in AL-CA and HCM patients by 3D-STE.
心脏淀粉样变性（CA）是一种罕见的全身性疾病，其特征为蛋白质衍生的纤维在细胞外沉积，而肥厚型心肌病（HCM）在组织病理学上的特征为心肌细胞肥大、排列紊乱、间质纤维化以及壁内小冠状动脉发育异常。本研究的目的是通过三维（3D）斑点追踪超声心动图（STE）比较轻链（AL）型CA和HCM患者左心房（LA）的容积和功能特征。
AL-CA组最初有17例AL-CA患者，但1例因图像质量不佳被排除，因此研究人群包括16例患者（平均年龄：64.0±9.6岁，5例男性）。将他们的结果与20例年龄匹配的HCM患者（平均年龄：59.8±5.2岁，10例男性）以及16例年龄匹配的健康对照者（平均年龄：58.2±7.2岁，6例男性）的数据进行比较。所有病例均进行了完整的二维多普勒超声心动图和3D-STE检查。
与对照组相比，AL-CA和HCM患者的LA容积均显著增加。与对照组相比，仅发现AL-CA患者的主动心房排空分数显著降低。与对照组相比，AL-CA患者的整体峰值和平均节段圆周、纵向及面积应变均显著降低，但HCM患者与对照组之间仅平均节段纵向峰值应变存在显著差异。虽然HCM患者与对照组在心房收缩时的整体和平均节段应变无差异，但与对照组和HCM患者相比，AL-CA患者的某些应变参数降低。
3D-STE显示AL-CA和HCM患者的LA功能特征模式不同。
